Elsevier

Heart Rhythm

Volume 4, Issue 10, October 2007, Pages 1292-1299
Heart Rhythm

Original-clinical
Relationship between arrhythmogenesis and disease activity in cardiac sarcoidosis

https://doi.org/10.1016/j.hrthm.2007.06.006Get rights and content

Background

In patients with cardiac sarcoidosis, ventricular arrhythmias and/or conduction disturbances are frequently observed and sometimes fatal. However, few reports on disease activity and arrhythmic events in cardiac sarcoidosis are available.

Objective

The purpose of this study was to investigate the relationship between disease activity and arrhythmic events in cardiac sarcoidosis and the effect of corticosteroid therapy.

Methods

The study population consisted of 15 cardiac sarcoidosis patients with new-onset symptomatic arrhythmia, including eight patients admitted once for complete atrioventricular block (CAVB), five patients admitted once for sustained ventricular tachycardia (VT), and two patients admitted twice for two arrhythmic events (one for CAVB and the other for sustained VT). Disease activity was evaluated by gallium-67 citrate (Ga) scintigraphy. All patients with positive Ga uptake were treated with corticosteroids, and arrhythmic events were evaluated by repeat Holter recordings.

Results

Positive uptake of Ga was observed in 8 (80%) of the 10 CAVB events and in 1 (14%) of the 7 sustained VT events (80% vs 14%, P = .02). Corticosteroids abolished myocardial Ga uptake in all nine patients with positive Ga uptake. After corticosteroid therapy was started, AV conduction improved in 5 of 9 CAVB patients (including 8 patients with new-onset CAVB and one patient with history of CAVB). However, ventricular arrhythmias were not improved after corticosteroid therapy.

Conclusion

In cardiac sarcoidosis patients, CAVB develops mainly during the active phase of the disease. Early treatment with corticosteroids might improve AV conduction disturbance. However, sustained VT is not closely linked with disease activity and frequently develops in the advanced stage of disease.

Introduction

Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Clinical manifestations and causes of death vary in different geographic regions.1, 2, 3 Cardiac involvement occurs in 20% to 27% of sarcoidosis patients in the United States and may occur in as many as 58% of patients in Japan.4, 5, 6 Manifestations of cardiac sarcoidosis include ventricular aneurysms, congestive heart failure, pericarditis, pericardial effusion, conduction abnormalities, and arrhythmias.7 Complete atrioventricular block (CAVB), ventricular arrhythmias, and congestive heart failure occur frequently and can be fatal. Ventricular tachycardia (VT) and CAVB are presumed to be the cause of sudden cardiac death in many patients with sarcoidosis. Thus, prevention and early treatment of these severe arrhythmic events are important for improving the prognosis.8 A better understanding of the underlying arrhythmogenic mechanism in cardiac sarcoidosis would be beneficial.

In cardiac sarcoidosis patients with left ventricular dysfunction, sustained VT occurs and frequently is inducible at electrophysiologic study. The mechanism of sustained VT in cardiac sarcoidosis is presumed to be reentry related to myocardial scar tissue.9 On the other hand, conduction abnormalities result from involvement of the basal intraventricular septum due to sarcoid granuloma or scar tissue.6, 10

Gallium-67 citrate (Ga) scintigraphy not only is a useful means for diagnosing cardiac sarcoidosis but also is useful in the assessment of disease activity of sarcoidosis.11, 12, 13, 14 However, few reports on the relationship between disease activity and arrhythmic events are available. Accordingly, we investigated the relationship between disease activity and occurrence of severe arrhythmic events in cardiac sarcoidosis and the effect of corticosteroid therapy on arrhythmia in the active cardiac sarcoidosis phase.

Section snippets

Study population and diagnostic criteria

Between July 1995 and August 2005, 15 patients diagnosed with cardiac sarcoidosis underwent cardiac assessment in our institution, including 12-lead ECG, ultrasound cardiography, coronary angiography, and left ventriculography. Diagnostic criteria for cardiac sarcoidosis established by the Specific Diffuse Pulmonary Disease Research Group, Sarcoidosis Division of the Japanese Ministry of Health, Labor, and Welfare were used.15 The criteria include category 1—histologic confirmation of

Patient characteristics

A total of 15 patients (8 men and 7 women; mean age 56 ± 13 years) were evaluated (Table 1). Reasons for hospitalization in all patients were CAVB or sustained VT. Clinically documented arrhythmic events were CAVB (n = 10), VPCs, and sustained VT (n = 7). According to the diagnostic criteria, category 1 findings were found in 6 patients, with findings based on endomyocardial biopsy in five patients and findings based on postmortem examination in one patient (no. 14). Average age at onset of

Discussion

In this study, we investigated the relationship between disease activity and arrhythmic events in cardiac sarcoidosis, focusing on new-onset symptomatic arrhythmia and the effect of corticosteroid therapy.

Conclusion

In cardiac sarcoidosis patients, CAVB develops mainly in the active phase, and early treatment with a corticosteroid might improve the AV conduction disturbance. In contrast, sustained VT is not closely linked with disease activity and frequently develops in the advanced stage of disease.

References (31)

  • K. Iwai et al.

    Racial difference in cardiac sarcoidosis incidence observed at autopsy

    Sarcoidosis

    (1994)
  • A. Perry et al.

    Causes of death in patients with sarcoidosisA morphologic study of 38 autopsies with clinicopathologic correlations

    Arch Pathol Lab Med

    (1995)
  • K.J. Silverman et al.

    Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis

    Circulation

    (1978)
  • Y. Matsui et al.

    Clinicopathological study of fatal myocardial sarcoidosis

    Ann N Y Acad Sci

    (1976)
  • R.L. Shammas et al.

    Sarcoidosis of the heart

    Clin Cardiol

    (1993)
  • Cited by (0)

    View full text